Cca

Cholangiocarcinoma
Synonyms	Bile duct cancer
Pronounce	N/A
Specialty	N/A
Symptoms	Jaundice, abdominal pain, weight loss
Complications	Liver failure, metastasis
Onset	Typically in older adults
Duration	Long-term
Types	N/A
Causes	Primary sclerosing cholangitis, liver fluke infection, hepatitis B, hepatitis C
Risks	Chronic liver disease, cirrhosis, obesity
Diagnosis	Imaging studies, biopsy
Differential diagnosis	N/A
Prevention	N/A
Treatment	Surgery, chemotherapy, radiation therapy
Medication	N/A
Prognosis	Generally poor
Frequency	Rare
Deaths	N/A

Cholangiocarcinoma (CCA) is a type of cancer that forms in the bile ducts, which are the slender tubes that carry the digestive fluid bile.

Classification

Cholangiocarcinoma is classified based on its location within the bile duct system:

• Intrahepatic cholangiocarcinoma: Occurs in the bile ducts within the liver.
• Perihilar cholangiocarcinoma: Also known as Klatskin tumor, occurs at the junction where the left and right hepatic ducts join.
• Distal cholangiocarcinoma: Occurs in the bile ducts outside the liver, closer to the small intestine.

Epidemiology

Cholangiocarcinoma is a rare form of cancer, with an incidence of approximately 1-2 cases per 100,000 people annually. It is more common in Southeast Asia due to the prevalence of liver fluke infections.

Etiology

Several risk factors have been associated with the development of cholangiocarcinoma:

• Primary sclerosing cholangitis
• Chronic liver disease and cirrhosis
• Hepatitis B and hepatitis C infections
• Liver fluke infections, particularly Opisthorchis viverrini and Clonorchis sinensis
• Obesity and diabetes mellitus
• Exposure to certain chemicals such as thorotrast

Pathophysiology

Cholangiocarcinoma arises from the epithelial cells of the bile ducts. The exact mechanism of carcinogenesis is not fully understood, but chronic inflammation and bile stasis are thought to play a significant role. Genetic mutations, such as those in the KRAS, BRAF, and IDH1 genes, have been implicated in the pathogenesis of cholangiocarcinoma.

Clinical Presentation

Symptoms of cholangiocarcinoma can vary depending on the location of the tumor but often include:

• Jaundice
• Abdominal pain
• Weight loss
• Pruritus
• Fever

Diagnosis

The diagnosis of cholangiocarcinoma typically involves a combination of imaging studies and biopsy:

• Ultrasound and CT scan: To visualize the bile ducts and identify any masses.
• MRI and MRCP: To provide detailed images of the bile duct system.
• Endoscopic retrograde cholangiopancreatography (ERCP): To obtain tissue samples for biopsy.
• Tumor markers: Elevated levels of CA 19-9 may be indicative of cholangiocarcinoma.

Treatment

The treatment of cholangiocarcinoma depends on the stage and location of the tumor:

• Surgical resection: The only potentially curative treatment, often involving hepatectomy or bile duct resection.
• Liver transplantation: Considered in select cases of early-stage perihilar cholangiocarcinoma.
• Chemotherapy: Common regimens include gemcitabine and cisplatin.
• Radiation therapy: Used as an adjunct to surgery or for palliation.

Prognosis

The prognosis for cholangiocarcinoma is generally poor, with a 5-year survival rate of less than 20%. Early detection and surgical resection offer the best chance for long-term survival.

Prevention

Preventive measures include:

• Avoiding liver fluke infections by consuming properly cooked fish.
• Managing chronic liver diseases and hepatitis infections.
• Regular monitoring for individuals with primary sclerosing cholangitis.

See also

• Liver cancer
• Pancreatic cancer
• Gallbladder cancer

External links

• American Cancer Society: Bile Duct Cancer
• National Cancer Institute: Bile Duct Cancer Treatment